Assessing PH Burden and Care Access

We developed a questionnaire containing questions intended to be answered by the parent of a child living with PH. The survey assessed age at PH diagnosis, congenital heart defects, presence of Down syndrome, maternal age at birth, gestational age, PH therapies utilized, and side effects attributed to PH medications (Treprostinil, Tadalafil, Sildenafil, Ambrisentan, Bosentan, Macitentan, Selexipag, and Riociguat) used during the past 6 months. We also queried participants’ knowledge, attitudes, and perceptions of the Potts Shunt procedure and combined heart/lung transplant. We also assessed specific access to care issues and care provider characteristics such as where PH care is received (local pediatrician’s office, specialty children’s hospital, no specialty PH care available), the type of PH care provider (pediatric cardiologist, pediatric pulmonologist, PH specialist, combination of cardiologist and pulmonologist), distance traveled (miles) to primary PH provider, travel time to PH provider (minutes), need to move residence to receive PH care for child (yes/no), whether genetic testing was offered at diagnosis (yes/no), whether genetic testing was performed (yes/no) and whether a genetic counselor discussed any genetic testing results (yes/no). Finally, we queried family sociodemographics including race (white/other), relationship to child living with PH (father, mother, guardian, primary caregiver), U.S. citizenship (yes/no), respondents’ educational attainment (high school, some college, associate’s degree, bachelor’s degree, graduate or professional degree), employment status (employed, homemaker, unemployed), household income (< $35,000, $35,000–74,999, > $75,000), and health insurance status (yes/no). The average time to complete the survey was 19.5 min (range: 8 to 56 min).